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EURRAD 00015

European Journal of Radiology. 10 (1990) 38-41 Elsevier

Rectal duplications in children: a presentation of four cases

A.E. Boothroyd and M. Hall Christine Department of Radiology. The Hospital for Sick Children, Great Onnond Street, London, U.K.

(Received 12 May 1989; revised version received 25 July 1989; accepted 30 September 1989)

Key words: Rectum, cyst; Rectum, abnormality; Cyst, rectum

Abstract

Four cases with histologically proven duplication cysts of the rectum are presented. Two ofthese had communicating and two had closed rectal duplications.

Introduction

Rectal duplications represent a difficult diagnostic

problem. They are rare, accounting for only 3.2% of bowel duplications [ 11, may present with only vague clinical symptoms and may give rise to subtle or absent radiological signs.

Case 1

A 3-year-old girl, who was otherwise well, presented with a two year history of abdominal distension and constipation. An examination under anaesthetic, and rectal biopsy had previously been performed and no evidence of Hirschsprung’s disease had been found. A plain abdominal radiograph showed marked faecal loading on the left side of the abdomen (Fig. 1). A barium enema examination demonstrated a 25 cm tubular duplication of the colon extending from the terminal ileum to the rectum and which communicated proximally. This was best demonstrated on a delayed film (Fig. 2) which is often routinely performed in patients with constipation. An intravenous urogram in the same patient showed a left-sided hydronephrosis due to partial obstruction by the mass.

Address for reprints: Dr. A.E. Boothroyd, Department of Radiology, The Middlesex Hospital, Mortimer Street, London W.C.1, U.K. Fig. 1. Plain abdominal radiograph demonstrating faecal loading.

0720-048X/90/%03.50 0 1990 Elsevier Science Publishers B.V. (Biomedical Division)

Fig. 2. Delayed film following a barium enema which shows barium retained within duplicated bowel.

Following excision of the duplication cyst marked clinical improvements were noted with cessation of symptoms and improved appetite and weight gain.

Fig. 3. Barium enema demonstrating a rectal duplication which extends to the splenic flexure.

normal. He was well post-operatively and has returned to Yugoslavia for follow-up.

Case 3 Case 2

A 5-year-old boy from Yugoslavia presented with a history of constipation and soiling since infancy. He was the product of a full-term normal delivery and was otherwise well. He was noted to have a rectal prolapse at 6 weeks which reduced spontaneously after passing a stool. At the age of 2 years a rectal operation had been performed, but no details of this are known. Diagnoses of anal stenosis, slow transit syndrome and a hypotonic rectal ampulla were suggested and the patient was referred to the Hospital for Sick Children for further investigation. An examination under anaesthetic revealed a pararectal mass which was incised and drained into the rectum. Subsequent barium enema examination showed a tubular duplication communi- cating below, through the surgical defect in the rectum and above with the splenic flexure (Fig. 3). Renal ultra- sound and micturating cystogram examinations were

A male infant (46 XY), the product of a full-term normal delivery, presented at birth with a hindgut mal- formation. He had ambiguous genitalia with perineal hypospadias and a bifid scrotum. The presence of a perineal mass extending from the bulbous urethra to the rectum raised the suspicion of a duplication cyst. Plain radiology revealed segmentation defects in the thora- columbar spine (Fig. 4). A barium enema examination was normal, as was renal ultrasound. At operation, on the 4th day of life, a cyst lined by large bowel mucosa was found which was consistent with a closed rectal duplication. The patient has had no further bowel problems during 11 years follow-up.

Case 4

A g-year-old boy presented with a history of soiling and constipation since infancy with intermittent ab-

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dominal pain and distension and occasional rectal pro- lapse. He had undergone multiple examinations under anaesthetic and had a normal rectal biopsy. He was

Fig. 4. Thoracic spine radiograph demonstrating segmentation de- The histological criteria for the diagnosis of bowel fects in the lower thoracic spine. duplication are the presence of gastrointestinal epithe-

TABLE I

Summary of clinical and radiological findings in four cases

referred to the Hospital for Sick Children because his weight had dropped below the tenth centile. A barium enema examination performed at the age of 6 years was normal. On this admission a left-sided pararectal mass was found on examination under anaesthetic and was incised through the rectum. Histology revealed a closed duplication cyst. He is currently well and no longer requires laxatives.

Discussion

Rectal duplication is a rare congenital abnormality; a survey of the world literature of cases of tubular colonic duplication [2] revealed only 53 cases between 1876 and 1981. Eighty per cent of these patients had other anomalies, notably genital and bladder duplica- tions. However, although rare, it is a diagnosis which should not be overlooked, since serious complications may develop. Perforation and haemorrhage are typically associated with ectopic gastric mucosa within the duplication. Obstruction, volvulus, intussusception and infection may also occur. Less severe complications, such as constipation and abdominal distension, may account for a very significant morbidity, as our three patients with a delayed presentation demonstrate. Severe cases have persisted into adulthood [3], where symptoms of constipation have been life-long. Another risk of failing to detect these duplications is the con- siderable risk of malignant change. Gibson et al. [4] reported carcinoma in 19% and a case of carcinoid has also been documented [ 51.

Case Age at diagnosis Sex Symptoms Nature of duplication Abnormality on barium enema

1 3 years F Abdominal distension and constipation

Communicating +

2 5 years M Constipation Soiling

Communicating +

3 Birth M Multiple GU and anorectal anomalies

Closed _

4 9 years M Abdominal distension and constipation

Closed -

lium, a layer of smooth muscle in the wall of the cyst and attachment to the alimentary tract [ 61. The four cases described fulfill the histological criteria for a rectal duplication.

Radiological demonstration of the duplication may be difficult. In two of our cases, even when a duplication cyst was clinically suspected a barium enema examina- tion was normal (cases 3 and 4). Both of these cases represented closed duplications. CT is particularly valuable in assessing perirectal masses [7,8]. It may demonstrate a communication with bowel and ac- curately locates the mass which may alter the surgical approach. Distinction from other causes of a pelvic cystic mass in a child such as a distended bladder, hydrometrocolpos, ovarian cyst, mesenteric cyst, cystic teratoma, urachal cyst and an anterior sacral meningo- coele may also be made, particularly if oral and intra- venous contrast are used.

A duplication cyst should be considered in the presence of an anorectal anomaly or in a child with a history of constipation, when Hirschsprung’s disease has been excluded and which is unresponsive to con- ventional management. A barium enema should be the prime investigation, but CT of the pelvis may prove

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diagnostic following a negative barium enema. Finally, it is important to assess the renal tract in all patients with a large bowel duplication to detect any associated congenital abnormalities of the urinary tract or any complications of the duplication cyst itself (case 1).

References

Dohn K, Povlsen OM. Enterocystomas: report of six cases. Acta Chir Stand 1951; 102: 21-35. Yousefzadeh DK, Bickers GH, Jackson JH, Benton C. Tubular colonic duplication-review of 1876-1981 literature. Paed Radio1 1983; 13: 65-71. Oluwole SF, Ademakinwa A. Rectal duplication in chronic large bowel obstruction. Can J Surg 1987; 30: 419-20. Gibson TC, Edwards JM, Shatiq S. Carcinoma arising in a rectal duplication cyst. Br J Surg 1986; 73: 377. Rubin SZ, Mancer JR, Stephens CA. Carcinoid in a rectal duplication: a unique paediatric surgical problem. Can J Surg 1981; 24: 351-352. McLeod JH, Purves JK. Duplications of the rectum. Dis Colon Rectum 1970; 13: 133-137. Panicek DM, Leeson SH, Hitch DC, Farrar FM. Perirectal mucocoele after imperforate anus repair. Paediatr Radio1 1987; 17: 73-74. Burchall MC. Radiologic impact on operative access to retrorectal cyst. Dis Colon Rectum 1987; 30: 396-397.